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Thrombocytopenia associated with galsulfase treatment

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dc.contributor.authorDogan, Murat
dc.contributor.authorCesur, YAŞAR
dc.contributor.authorPEKER, Erdal
dc.contributor.authorÖner, Ahmet Cihat
dc.contributor.authorDOGAN, Sekibe Zehra
dc.contributor.institutionauthorCESUR, YAŞAR
dc.date.accessioned2020-10-30T00:24:27Z
dc.date.available2020-10-30T00:24:27Z
dc.date.issued2011-07-01T00:00:00Z
dc.description.abstractMucopolysaccharidosis type VI (MPS VI), or Maroteaux-Lamy syndrome, is a lysosomal storage disorder that results from a deficiency of the enzyme N-acetylgalactosamine-4-sulfatase or arylsulfatase B (ASB). It is a relatively rare disorder, with an estimated incidence of 1 in 248,000 to 1 in 300,000. The diagnosis is made on the basis of findings of elevated urine glycosaminoglycans and a deficiency of ASB activity in leukocytes or cultured fibroblasts. In treatment of MPS VI, enzyme replacement therapy (galsulfase; human recombinant ASB enzyme) became available. Infusions of galsulfase were generally well tolerated. But in some patients, infusion-associated reactions including rash, urticaria, headache, hypotension, nausea, and vomiting were documented and were managed successfully by interrupting or slowing the rate of infusion and/or by the administration of antihistamines, antipyretics, corticosteroids, or oxygen. Here, we report a case with MPS VI who developed thrombocytopenia after third dose of therapy. To the best of our knowledge, this is the first report about thrombocytopenia associated with galsulfase therapy in the literature. Additionally, with this report, we want to share our approach for this case.
dc.identifier.citationDogan M., Cesur Y., PEKER E., Öner A. C. , DOGAN S. Z. , -Thrombocytopenia associated with galsulfase treatment-, HUMAN & EXPERIMENTAL TOXICOLOGY, cilt.30, ss.768-771, 2011
dc.identifier.doi10.1177/0960327110379023
dc.identifier.scopus79959735952
dc.identifier.urihttp://hdl.handle.net/20.500.12645/27222
dc.identifier.wosWOS:000291891200024
dc.titleThrombocytopenia associated with galsulfase treatment
dc.typeArticle
dspace.entity.typePublication
local.avesis.idf6964585-cb8b-401b-be1c-4e08a0dcbf6c
local.publication.isinternational1
relation.isAuthorOfPublicationddd881a5-c7cb-41e5-b4e1-0693a98a0d29
relation.isAuthorOfPublication.latestForDiscoveryddd881a5-c7cb-41e5-b4e1-0693a98a0d29
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